Emergency surgery due to diaphragmatic hernia: case series and review

A systematic review was performed by consulting PubMed/MEDLINE from 1983 to 2017 using the terms “emergency surgery”, associated with “traumatic diaphragmatic rupture”, and “congenital diaphragmatic hernia”. The search returned 555 papers (Fig. 7). Three hundred twenty-three publications were excluded because these articles were not written in English (N = 87), presented cases in childhood (<19 years old; N = 178) or were not interesting human species (N = 58); 32 papers were excluded because regarded hiatal hernia, 40 paraesophageal hernia and 59 elective setting. Consequently, the full texts of 101 articles were assessed for eligibility: the ethiopathogenesis was traumatic in 697 patients and congenital in 38 (Table 1).

Diaphragmatic rupture with abdominal organ herniation was first described in 1541 by Sennertus [11]. Congenital diaphragmatic hernias are prenatally or during the neonatal period diagnosed. On the contrary, CDH in adulthood are exceedingly rare and can occur through an anterior parasternal Morgagni foramen or through a posterolateral, mainly left-sided, named as Bochdalek hernia, firstly described in 1848 [12]. The aetiology is still under study, but the disease is due to the failure of closure of the canal between the septum transversum and the oesophagus during the 8th week of gestation. Morgagni hernia is a rare disease caused by the defective development of the sternal attachments to the diaphragm. Traumatic diaphragmatic hernias are thought to be produced by a sudden increase in the pleuroperitoneal pressure gradient occurring at areas of potential weakness along embryological points of fusion [13].

DR usually result from blunt or penetrating injuries or iatrogenic causes and result in entry of an abdominal hollow viscus or the omentum into the pleural cavity, which may lead to incarceration and even strangulation with a fatal outcome. Traumatic diaphragmatic hernias are frequently caused by a penetrating injury (10–19%), sometimes by blunt thoracic-abdominal trauma (5%) [14, 15]. Moreover, some authors described rare and particularly cases of DR after surgery or pregnancy; that is Sano A. et al. reported a case of a pregnant woman in the 28th week of pregnancy, who was underwent to emergency caesarean section and repair of the diaphragm [16]; Moussa G. et al., described a right DR in a patient with previous history of window fenestration and sarcoidosis [17]; Nakamura T. et al., reported a case of right DR in patient with a history of hepatic carcinoma treated with radiofrequency ablation [18]. Furthermore, there was an association between Marfan’s syndrome and CDH as Barakat et al. reported [19].

CDH formation is found 80% on the left side [20]. Also, 88–95% of diaphragmatic ruptures occurred on the left side [21], especially, blunt trauma causes large diaphragmatic defects, commonly involving (>80%) the left posterolateral diaphragm [22]. The right haemidiaphragm is stronger than the left one because of the size of the liver which has a protective effect. For this reason, the side ruptures are very rare and associated with high mortality and morbidity rate [23].

The review of literature reported in this study confirmed the high frequency of left defect 80%, and only two cases of bilateral DR were reported.

Nayak et al. described severe symptoms, in 46% of CDH cases with 32% of mortality due to visceral strangulation [24]. Moreover, the literature analysis shows a variable rate of delayed symptoms (5–45.5%) [25, 26]. Late-presenting CDH of left sided typically produces acute, obstructive, gastrointestinal symptoms, chronic dyspnea, chest pain, recurrent abdominal pain, postprandial fullness and vomiting, evolving to cardiorespiratory failure [27]. Indeed, right-sided CDH is usually associated with only respiratory issues because partial liver displacement may block the further herniation of hollow viscera [1]. Although the presence of bowel sounds within the chest and the absence of breath sounds are typical findings associated with a CDH, a misdiagnosis rate of 38% has been reported [28]. Obviously, in totally asymptomatic cases, diagnosis is very hard. On the contrary, when acute presentations occur because of the increasing of abdominal pressure and consequent rapid visceral displacement into the chest or due to rapid distension of previously herniated viscera, diagnosis is clear [29, 30]. Chest X-ray and barium studies are useful for determining which viscera have herniated into the thorax. The most common reported radiological finding of CDH is the opaqueness of the hemithorax usually associated with mediastinal shift to the contralateral side. Moreover, the position of the nasogastric tube in the chest cavity will provide an important indicator and prompt correct diagnosis. Computed tomography can be considered the gold standard technique for diagnosis, offering the unique opportunity to evaluate the presence, size and location of a diaphragmatic defect, as well as the contents of various types of diaphragmatic hernias [31] and showing sensitivity and specificity of 14–82% and 87%, respectively [32]. MRI is also useful, but usually it is not performable in emergency. However, it is usually employed in stable patients or where the CT scan is equivocal [33]. According with literature, in this reported experience, a definitive diagnosis was made with CT scan and barium studies.

Late-presenting CDH is considered as a benign condition but it can rapidly becomes a life-threatening disease [1, 27, 28, 31, 33]; consequently, an immediate surgical treatment is mandatory. Associated anomalies in late-presenting CDH patients, such as congenital heart disease, Fryns syndrome and trisomy 18, have been reported in 8.6–80% of cases [1, 2, 27, 28], significantly increasing the mortality rate. At this proposal, in case 1, even if there was a high suspicion of congenital syndrome, surprisingly it was not confirmed by genetic studies.

Surgical repair typically involves primary or patch closure of the diaphragm through an open abdominal approach. When the diagnosis is delayed, due to suspicions of adhesions between viscera and chest, thoracotomy or combined thoracic-abdominal approach is preferred, as in the reported case 2. Some authors have reported success with thoracoscopic approach but vitiated by an increased incidence of hernia recurrence [34‐36]. Furthermore, during thoracoscopy, an intraoperative pulmonary hypertension with subsequent hemodynamic instability could develop; moreover, the placement and management of a patch results in substantially longer operating times. For these reasons, thoracoscopic repair of CDH is preferred in the presence of small diaphragmatic defects and/or mild pulmonary hypertension [37]. Nowadays, the laparoscopic approach is safe and feasible for CDH and it could be an excellent option [37], as in case 3.

However, emergency surgery is the treatment of choice for diaphragmatic rupture. In delayed cases, thoracic approach is recommended to reduce viscera-pleural adhesions and to avoid intra-thoracic visceral perforation with catastrophic complications [38]. When the suspicion of intestinal obstruction is evident, an abdominal approach may also be required to control organs. Although the type of closure used for diaphragmatic hernias is still a matter of debate, it is generally accepted that most defects can be primarily closed with a non-absorbable suture [39]. Mesh repair usually is used when the defect is too large to be primarily closed and the use of tension free mesh is vital to the success of the procedures. Recently, biologic mesh has been introduced to replace the synthetic one because of its lower rate of hernia recurrence, higher resistance to infections and lower risk of displacement [7, 40]; however, limited evidence in literature yet exists about their superiority. Indeed, in our previous experience, biologic meshes have also been used in contaminated surgical fields with favourable results [40]. However, because of the rarity of this condition, clinicians should be encouraged to publish their experience with biologic meshes in diaphragmatic hernia repair [7].